Liebross R H, Ha C S, Cox J D, Weber D, Delasalle K, Alexanian R
Department of Radiation Oncology, The University of Texas, M.D. Anderson Cancer Centre, Houston 77030, USA.
Radiother Oncol. 1999 Sep;52(3):245-9. doi: 10.1016/s0167-8140(99)00114-0.
Solitary extramedullary plasmacytoma (EMP) represents a rare category of malignant disease on which there are limited data in regard to diagnosis, staging and natural history. This study attempted to clarify the clinical course of solitary extramedullary plasmacytoma after radiation or surgical therapy given with curative intent.
The diagnosis was based on a mass of clonal plasma cells separate from bone or bone marrow without evidence of occult disease elsewhere. Between 1963 and 1996, 22 previously untreated patients with an EMP were diagnosed. Disease presented in the head or neck in 86%, usually in the nasal cavity (NC) or maxillary sinus (MS), and in these areas local bone destruction was found in 10 of 11 patients. Among all patients, serum myeloma protein was present in three patients (14%) and Bence Jones protein alone was found in two patients (9%). Radiation therapy was the sole treatment for 18 of 22 patients, and the median radiotherapy dose was 50 Gy (range, 40-60 Gy); five of seven patients with an EMP of oral cavity (OC), oropharynx (OP), nasopharynx (NP), parotid or larynx also received elective neck irradiation. Two patients underwent surgery plus postoperative irradiation of a plasmacytoma of the sigmoid colon or pleura, and two patients had resection alone of a plasmacytoma of the colon or cervical lymph node.
Local control was achieved in 21 of 22 patients (95%), and disease never recurred in regional nodes. Disappearance of myeloma protein occurred in three of five patients with an evaluable abnormality. Multiple myeloma developed in seven patients (32%), all within 5 years. The 5-year rate of freedom from progression to multiple myeloma was 56% and the median survival was 9.5 years.
Radiation therapy achieved excellent locoregional control of EMP with an approximate cure fraction of 50%.
孤立性髓外浆细胞瘤（EMP）是一种罕见的恶性疾病，关于其诊断、分期及自然病程的数据有限。本研究旨在阐明以治愈为目的进行放疗或手术治疗后孤立性髓外浆细胞瘤的临床病程。
诊断依据为一团与骨骼或骨髓分离的克隆性浆细胞，且无其他部位隐匿性疾病的证据。1963年至1996年期间，22例既往未接受治疗的EMP患者被确诊。86%的疾病发生于头颈部，通常在鼻腔（NC）或上颌窦（MS），在这些部位，11例患者中有10例发现局部骨质破坏。所有患者中，3例（14%）存在血清骨髓瘤蛋白，2例（9%）仅发现本周氏蛋白。22例患者中有18例仅接受放射治疗，中位放疗剂量为50 Gy（范围40 - 60 Gy）；7例口腔（OC）、口咽（OP）、鼻咽（NP）、腮腺或喉部EMP患者中的5例也接受了选择性颈部照射。2例患者接受了乙状结肠或胸膜浆细胞瘤的手术及术后放疗，2例患者仅对结肠或颈部淋巴结的浆细胞瘤进行了切除。
22例患者中有21例（95%）实现了局部控制，区域淋巴结未出现疾病复发。5例可评估异常的患者中有3例骨髓瘤蛋白消失。7例患者（32%）发生了多发性骨髓瘤，均在5年内。进展为多发性骨髓瘤的5年无进展率为56%，中位生存期为9.5年。
放射治疗对EMP实现了良好的局部区域控制，近似治愈率为50%。
